ANIMAL STUDIES OF CYSTIC-FIBROSIS

Cystic fibrosis is the most common life-threatening autosomal recessiv e genetic disorder in Caucasian populations, It is a disease primarily of epithelial tissues, including the airway, pancreatic duct, intesti ne, genital tract and sweat glands, The affected gene was cloned and c haracterized in 1989, In the absence of an identified natural animal m odel of the disease, a major effort has been made to develop transgeni c cystic fibrosis mice, by disrupting the gene in these laboratory ani mals, Such mice show many, but not all, of the symptoms of cystic fibr osis. In this article, the major past and present contributions of oth er animal systems to our understanding of cystic fibrosis are examined and their potential for future studies of this disease are discussed, It is intended to give the reader a broad overview of the field, expl oring the usefulness of animal studies, rather than dealing more fully with specific aspects of cystic fibrosis.